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A prior drug trial of cannabidiol for treatment-resistant epilepsy in patients with Sturge-Weber syndrome (SWS), a rare neurovascular condition, implicated improvements in neurological, quality of life (QOL), neuropsychologic, psychiatric, and motor outcomes.Ten subjects with SWS brain involvement, controlled seizures, and cognitive impairments received study drug in this Johns Hopkins institutional review board-approved, open-label, prospective drug trial. Oral cannabidiol was taken for six months (dose ranged from 5 to 20 mg/kg/day). SWS neuroscore, port-wine birthmark score, QOL, and adverse events were recorded every four to 12 weeks. Neuropsychologic, psychiatric, and motor assessments were administered at baseline and six months' follow-up. Most evaluations were conducted virtually due to the coronavirus disease 2019 pandemic.Cannabidiol was generally well tolerated. Six subjects reported mild to moderate side effects related to study drug and continued on drug; one subject withdrew early due to moderate side effects. No seizures were reported. Significant improvements in SWS neuroscore, patient-reported QOL, anxiety and emotional regulation, and report of bimanual ability use were noted. Migraine QOL scores were high at baseline in these subjects, and remained high. Neuropsychologic and other QOL and motor outcomes remained stable, with some within-subject improvements noted.Further studies are needed to determine whether Epidiolex can improve quality of life and be beneficial for neurological, anxiety, and motor impairments in SWS independent of seizure control. Large multicentered studies are needed to extend these preliminary findings.

Background Sturge-Weber syndrome is a rare neurovascular disorder associated with capillary malformation, seizures, cognitive impairments, and stroke-like episodes (SLEs), arising from a somatic activating mutation in GNAQ. Studies suggest this mutation may cause hyperactivation of the mammalian target of rapamycin pathway. Sirolimus is an mammalian target of rapamycin inhibitor studied in other vascular anomalies and a potentially promising therapy in Sturge-Weber syndrome. Methods Ten patients with Sturge-Weber syndrome brain involvement and cognitive impairments were enrolled. Oral sirolimus was taken for six months (maximum dose: 2 mg/day, target trough level: 4-6 ng/mL). Neuropsychological testing, electroencephalography, and port-wine score were performed at baseline and after six months on sirolimus. Neuroquality of life, adverse events, and Sturge-Weber Syndrome Neurological Score (neuroscore) were recorded at each visit. Results Sirolimus was generally well tolerated; one subject withdrew early. Adverse events considered related to sirolimus were mostly (15/16) grade 1. A significant increase in processing speed was seen in the overall group (P = 0.031); five of nine patients with available data demonstrated statistically rare improvement in processing speed. Improvements were seen in the neuroquality of life subscales measuring anger (P = 0.011), cognitive function (P = 0.015), and depression (P = 0.046). Three subjects experiencing SLEs before and during the study reported shortened recovery times while on sirolimus. Conclusions Sirolimus was well tolerated in individuals with Sturge-Weber syndrome and may be beneficial for cognitive impairments, especially in patients with impaired processing speed or a history of SLE. A future, randomized, placebo-controlled trial of sirolimus in patients with Sturge-Weber syndrome is needed to further understand these potentially beneficial effects.

Patients with myelomeningocele-type spina bifida are at increased risk of developing kidney disease from neurogenic bladder. Differences between creatinine- and cystatin C-estimated glomerular filtration rates were examined in patients with thoracic versus sacral level myelomeningocele given presumed differences in muscle mass.A retrospective chart review (2005-2018) was performed on 57 adults with myelomeningocele [thoracic n = 44 (77%); sacral n = 13 (23%)]. Concurrently obtained creatinine and cystatin C levels were extracted and calculated creatinine- and cystatin C-estimated glomerular filtration rates were compared.Mean creatinine-estimated glomerular filtration rate was significantly higher for thoracic [140.8 ml/min (SD = 23.9)] versus sacral myelomeningocele [112.0 ml/min (SD = 22.6), P = 0.0003]. There was no difference in cystatin C-estimated glomerular filtration rate between sacral [116.6 ml/min (SD = 23.7)] and thoracic myelomeningocele [124.8 ml/min (SD = 17.9)]. The mean difference between creatinine- and cystatin C-estimated glomerular filtration rates in thoracic myelomeningocele [24.2 ml/min (SD = 16.3)] was significantly greater than in sacral myelomeningocele [-12.8 (SD = 15.7), P0.0001].There was a significantly higher discrepancy between creatinine- and cystatin C-estimated glomerular filtration rates in thoracic versus sacral motor levels of myelomeningocele. These data suggest that creatinine-estimated glomerular filtration rate may overestimate kidney function in patients with thoracic myelomeningocele. Providers who manage patients with thoracic myelomeningocele should consider monitoring cystatin C to evaluate for underlying renal disease.

Parent-reported satisfaction is an important outcome measure in pediatric neuropsychology; however, self-report of patient experience following lengthy pediatric assessments has been underinvestigated. Written at a first grade reading level and utilizing touch screen and read-aloud functionality, a set of 15 operationally-focused items were administered to evaluate pediatric patient experience of neuropsychological and psychological assessment. One-hundred ninety-seven clinically-referred patients (M age = 11.74, SD = 3.17, range = 5.86–19.02 years, 56% male) answered the post-assessment survey. The majority of patients (n = 167; 84.77%) accurately completed the initial two validity items, even though many had parent-reported ratings of reading difficulty. More than a third of patients indicated that the assessment made them tired (39%), but fewer patients reported feeling bored (13%) or worried (7%) during testing. Moreover, most patients reported having fun (66%) and many indicated that testing was preferable to other activities typically performed at that same time (i.e., school, homework). Responses to items assessing the child’s interactions with the clinician and the child’s effort provided little variation and were answered in a socially desirable manner. Items that focused upon the child’s personal response to the assessment appointment (e.g., boredom, worry), however, elicited greater variance in patient responding. In general, patient responses did not suggest that assessment was an aversive experience. Overall, these proof of concept findings suggest that most referred pediatric patients, even those with learning issues, may be capable of independently navigating and completing self-report questionnaires while providing differential responses to items assessing clinical experience. Self-report questionnaires appear to be a feasible method for acquiring pediatric patient-reported experiences of assessment.

OBJECTIVE: The correct “dosing” of neuropsychological assessment is of interest for the purposes of cost management and the personalization of medicine/assessment. In this context, embedded IQ screening, rather than routine comprehensive IQ testing, may be useful in identifying youth at risk for Intellectual Disability (ID) for whom further assessment is needed. This retrospective, cross-sectional study examined subtests from the Wechsler Intelligence Scale for Children-Fifth Edition (WISC-5) needed to identify youth with Full Scale IQ (FSIQ) ≤75. METHOD: Data were obtained from a large pediatric clinically referred sample (N = 4,299; Mean Age = 10.7 years; Range = 6–16y; 66% male; 54% White; 29% receiving Public Insurance), divided into training (n = 2149) and test (n = 2150) samples. RESULTS: In the training sample, sequential and additive regression-based models for predicting FSIQ comprised of one (Block Design [BD]), two (BD + Similarities [SI]), three (BD + SI + Matrix Reasoning [MR]), and four (BD + SI + MR + Digit Span [DS]) subtests of the WISC-5 explained 61.3%, 82.7%, 88.5%, and 93.0% of FSIQ variance, respectively. Using a predicted FSIQ ≤ 80 as a cut score to identify persons with observed FSIQ ≤75, the two subtest (BD + SI) model showed strong sensitivity (83.4), specificity (90.5), and negative predictive value (96.2) in the test sample; however, positive predictive value was low (65.3%). Three and four subtest models provided incremental, but modest gains in classification metrics. CONCLUSIONS: Findings suggest the first several subtests of the WISC-5 can be used to identify clinically referred youth at risk for ID who subsequently require full administration of the WISC-5 for consideration of an ID diagnosis.

The present study examined clinical utility of teacher ratings on the Colorado Learning Difficulties Questionnaire (CLDQ) learning difficulties screening within a referred, school-aged sample ( N =519, 5-18 years). Of this sample, 419 youth had CLDQ reading scale scores from a reading or general education teacher, and 338 had CLDQ math scale ratings from a math or general education teacher. Sensitivity, specificity and receiver operating characteristic (ROC) curves were examined for specific reading and math difficulties (achievement SS

The present study examined the intellectual and adaptive functioning in a sample of children and young adults with Sturge-Weber Syndrome (SWS). A total of 80 research participants from a SWS study database underwent full neurological evaluation as part of their participation or concurrent medical care. Twenty-nine of the participants received neuropsychological evaluations. Analyses indicated no significant demographic or neurological differences between those who did and did not receive neuropsychological evaluations. Overall, the neuropsychological evaluation sample displayed significantly lower functioning relative to published normative data across domains of intellectual and adaptive functioning. Thirty-two percent of the sample displayed impaired performance (standard score ≤ 75) in intellectual functioning and 58% displayed impaired performance in adaptive functioning. Hemiparesis status independently predicted overall adaptive functioning while seizure frequency independently predicted overall intellectual functioning. Younger participants displayed significantly higher (more intact) ratings in adaptive functioning compared to older participants, specifically in overall adaptive functioning, motor skills, and community living skills. A composite measure of neurological status (SWS-NRS) incorporating seizure and hemiparesis status effectively distinguished between individuals with impaired or nonimpaired adaptive and intellectual functioning and showed promise as a screening method for identifying individuals with more involved intellectual and/or adaptive needs.

In children with neurodevelopmental disorders, it can be assumed that there has been early interference to the developing nervous system, which can take place during prenatal, perinatal, or postnatal development. There are two primary approaches to classification of neurodevelopmental disorders—one emphasizing behavior, and the other emphasizing neurology. This chapter describes the following four behaviorally defined neurodevelopmental disorders: intellectual developmental disorder, specific learning disorders, attention deficit/hyperactivity disorder, and autism spectrum disorder. Neuropsychologists search for neurological correlates of behaviorally defined conditions (ADHD, dyslexia, autism). At the same time, they search for behavioral phenotypes of genetic or neurologically defined disorders (e.g, Williams syndrome, Neurofibromatosis Type 1, Down syndrome). The primary role for the neuropsychologist when working with children with genetic and/or neurodevelopmental disorders is to help better understand the current fit between the child's unique biology and the ever-changing demands (and supports) in the environment.

Background Sturge-Weber syndrome is characterized by a facial port-wine birthmark, vascular eye abnormalities, and a leptomeningeal angioma. Attention and behavioral issues are common in Sturge-Weber syndrome. However, literature evidence for stimulant treatment is minimal. This study evaluates stimulant medication safety and efficacy in individuals with Sturge-Weber syndrome. Methods The research database of the Hunter Nelson Sturge-Weber Center (n = 210 subjects in the database) was reviewed for stimulant use. Twelve patients (mean age 10.5 years, age range 4 to 21 years) on stimulants were seen between 2003 and 2012. A retrospective chart review obtained comorbid diagnoses, stimulant type and dosage, medication side effects, vital signs, and medication efficacy. Results All 12 patients had brain involvement (unilateral, nine; bilateral, three). Additional comorbidities included epilepsy (twelve), hemiparesis (eight), headaches (eight), and vision deficits (six). Eight patients reported side effects, primarily appetite suppression (four) and headaches (three). There were no statistically significant changes in weight or blood pressure 6 months after medication initiation. Medication efficacy was subjectively reported in 11 patients. Seven patients remained on stimulants at their most recent follow-up visit. Conclusions This study preliminarily evaluates stimulant medication use in a small group of Sturge-Weber syndrome patients. Stimulants were tolerated and effective in most subjects. Side effects were mostly minor and medication did not negatively affect growth or vital signs. Stimulant medication may be a safe and effective intervention for Sturge-Weber syndrome children with attention issues/attention deficit hyperactivity disorder. Further studies with larger sample sizes are needed.

The Diagnostic and Statistical Manual of Mental Disorders—Fifth Edition (DSM-5) diagnostic criteria for intellectual disability (ID) include a change to the definition of adaptive impairment. New criteria require impairment in one adaptive domain rather than two or more skill areas. The authors examined the diagnostic implications of using a popular adaptive skill inventory, the Adaptive Behavior Assessment System–Second Edition, with 884 clinically referred children (ages 6–16). One hundred sixty-six children met DSM-IV-TR criteria for ID; significantly fewer (n = 151, p = .001) met ID criteria under DSM-5 (9% decrease). Implementation of DSM-5 criteria for ID may substantively change the rate of ID diagnosis. These findings highlight the need for a combination of psychometric assessment and clinical judgment when implementing the adaptive deficits component of the DSM-5 criteria for ID diagnosis.

Objective This study uses the Life Course Model for Spina Bifida (SB) to advance knowledge of factors associated with change in quality-of-life (QOL) among emerging adults with SB. Design Forty-eight participants (mean [SD], 22.04 [2.16] yrs) completed self-report questionnaires at two time points, 15 mos apart. Four QOL domains (physical health, psychological, social relationships, and environment) were measured using the World Health Organization QOL-BREF version. SB clinical data were collected via chart reviews. Paired t tests and reliable change indices evaluated group- and individual-level QOL change, respectively. Multiple regression analyses tested the contributions of the Life Course variables in explaining change in QOL over time. Results No significant group-level differences in the QOL domains were found between time 1 and time 2, but there was substantial individual variation in QOL over time. SB severity was related to a decline only in psychological QOL (B = -0.68, P = 0.02). Increased pain was associated with reduced physical health (B = -0.29, P = 0.049) and psychological (B = -0.29, P = 0.03) QOL at time 2, whereas greater family satisfaction was related to improved QOL in several domains. Conclusions Clinicians should be aware of the negative impact of pain and the protective influence of family satisfaction on QOL in emerging adults with SB.

Aim We examined the implications of using the Full Scale IQ (FSIQ) versus the General Abilities Index (GAI) for determination of intellectual disability using the Wechsler Intelligence Scales for Children, fourth edition (WISC-IV). Method Children referred for neuropsychological assessment (543 males, 290 females; mean age 10y 5mo, SD 2y 9mo, range 6–16y) were administered the WISC-IV and the Adaptive Behavior Assessment System, second edition (ABAS-II). Results GAI and FSIQ were highly correlated; however, fewer children were identified as having intellectual disability using GAI (n=159) than when using FSIQ (n=196). Although the 44 children classified as having intellectual disability based upon FSIQ (but not GAI) had significantly higher adaptive functioning scores than those meeting intellectual disability criteria based upon both FSIQ and GAI, mean adaptive scores still fell within the impaired range. FSIQ and GAI were comparable in predicting impairments in adaptive functioning. Interpretation Using GAI rather than FSIQ in intellectual disability diagnostic decision-making resulted in fewer individuals being diagnosed with intellectual disability; however, the mean GAI of the disqualified individuals was at the upper end of criteria for intellectual impairment (standard score 75), and these individuals remained adaptively impaired. As GAI and FSIQ were similarly predictive of overall adaptive functioning, the use of GAI for intellectual disability diagnostic decision-making may be of limited value.

The Behavior Assessment Scale for Children (BASC-2) is widely used to assess for internalizing/externalizing behavior problems; however, the role of its adaptive scales is not well established. This study examined the sensitivity of the parent version of the BASC-2 Adaptive Skills Composite (ASC) in identifying children with impairments on a more comprehensive measure of adaptive functioning, the Adaptive Behavior Assessment System (ABAS-II). Participants were referred for neuropsychological assessment (N = 1884, M age = 10.96 years, SD = 3.50, 65% male). The ASC, rather than individual BASC-2 adaptive scales, showed the strongest correlations with the ABAS-II General Adaptive Composite (GAC) and domains. Receiver operating characteristic analysis showed that the BASC-2 ASC discriminated children with impaired adaptive functioning on the ABAS-II (≥ -2 SD) from those without impairment (area under curve range = .86[social domain] to .78[practical domain]); however, sensitivity was inadequate at the typical -2 SD cut-off (48%). A -1 SD cut-off on the BASC-2 (T-score ≤ 40) was more appropriate for predicting impairment on the ABAS-II (89%). Among children with impaired IQ, the ABAS-II GAC was impaired in 69% compared to only 36% on the ASC. Findings have implications for the diagnosis of Intellectual Disability and for quantifying the functional impact of other diagnoses, particularly with the release of DSM-5.

Impairments in adaptive functioning are frequently associated with intellectual disability (ID); however, adaptive dysfunction can be seen in many individuals with a variety of neurological conditions without ID. The extent to which other variables may be associated with adaptive dysfunction is unclear. In a mixed clinical sample of children (n = 348) consecutively referred for neuropsychological evaluation, the majority were rated as showing weak adaptive skills (ABAS-II, >1 SD below the mean; 71%), with a substantial proportion evidencing frank impairment (>2 SD below the mean, 45%). We examined patterns of scores on measures of intelligence (WISC-IV) and behavioral/affective dysregulation (BRIEF and BASC-2). Using hierarchical cluster analysis, a four cluster model yielded the most appropriate fit and adaptive functioning was subsequently examined across clusters. As expected, adaptive functioning was most intact in the cluster characterized by average IQ and minimal behavioral dysregulation. Other clusters were marked by adaptive dysfunction and distinguished by sub-average intellectual functioning and varying behavioral/emotional dysregulation. In contrast to traditional views associating low IQ with adaptive dysfunction, adaptive impairment was comparable between the cluster characterized by low intelligence and the cluster with average intelligence but significant behavioral dysregulation. These data suggest that adaptive functioning should be considered across various cognitive/behavioral conditions. (JINS, 2013, 19, 1–9)

Sturge-Weber syndrome is a neurocutaneous disorder with skin, eye, and brain involvement. Prior series suggest about 50% of patients have seizures/neurodeterioration. Low-dose (3-5 mg/kg/d) aspirin use in this population is controversial. This study further addresses the side effects and outcomes of low-dose aspirin usage in Sturge-Weber syndrome. Fifty-eight subjects on aspirin with brain involvement were analyzed in a retrospective chart review. Charts were evaluated for brain involvement, age at first seizure, and side effects. Subjects' clinical stability was compared using neurologic scores. The majority of subjects had neurologic scores reflecting reasonable seizure control (91%), none or mild hemiparesis (57%), no vision impairment (71%), and none or mild cognitive impairment (80%). Forty-nine reported no significant side effects, and 9 reported either allergic reaction or minimal to significant bleeding on aspirin. This cohort's clinical experience adds significant support for low-dose aspirin use to optimize neurodevelopmental outcome in Sturge-Weber syndrome with minimal side effects.

Aim To advance understanding of the interrelationships of sex, level of lesion (LOL), self-management, community integration (employment, independent living), and quality of life (QOL) in young adults with myelomeningocele. Method A multicenter convenience sample of 50 individuals with myelomeningocele, 18 to 25 years of age (mean age 21y 5mo, SD 2y), participated in a structured clinical interview on self-management (Adolescent Self-Management and Independence Scale II [AMIS II]) and completed a self-report questionnaire comprising standardized measures. QOL was assessed using the World Health Organization Quality of Life (WHOQOL)-BREF instrument. A chart review yielded clinical data. Results Most participants were Caucasian (78%), female (56%: 28 females, 22 males), unemployed (58%), and in supervised living environments (74%). Eighty per cent had a history of hydrocephalus requiring shunt placement. A lumbar LOL was most frequently reported (64%), followed by a sacral LOL (22%), and thoracic LOL (7%). Males were more likely to report employment (p=0.008), but females had greater success in transitioning into independent living settings (p=0.015). LOL was a significant predictor of specific dimensions of self-management, employment, and QOL (p

This article outlines and summarizes the rationale and the working process that was undertaken by the National Spina Bifida Program to address the issues of transitioning throughout the life course for persons growing up with spina bifida. Their challenges include achieving independent living, vocational independence, community mobility, and participation in social activities, and health management. The creation, the underlying concepts, and the dissemination of the Life Course Model are described.

Sturge-Weber syndrome is a rare neurocutaneous disorder that often results in functional impairment caused by motor (typically hemiparesis) and cognitive deficits. A retrospective chart review of physiatric evaluation of 30 individuals, aged 4 mos to 55 yrs (median age, 2.4 yrs), with Sturge-Weber syndrome with brain involvement was conducted for the purpose of summarizing physiatric findings and recommendations in this cohort. Presence or absence of motor, cognitive, and behavioral concerns and need for orthoses, spasticity management, and therapy services were noted. Hemiparesis was common, but the need for intervention for spasticity was rare. Cognitive and behavioral concerns were noted frequently, meriting additional evaluation. Case vignettes are presented to highlight (1) a child with repeated functional setbacks in association with increased seizure frequency who, with seizure control, demonstrated return to functional baseline and subsequent further skill development and (2) a child with Sturge-Weber syndrome who made functional gains with constraint-induced movement therapy.

Objective Based on social ecological theory, this study was designed to examine the unique relationships between multi-level ecological factors and psychological symptoms in young adults with spina bifida (SB). Method A sample of 61 individuals with SB, 18–25 years of age, completed standardized self-report measures of attitude toward SB, satisfaction with family functioning, Chronic Care Model (CCM) services, and depressive and anxiety symptoms. A chart review yielded SB clinical data. Results High rates of depressive and anxiety symptoms were found. Hierarchical regression analysis identified the proximal individual (attitude toward SB) and family (satisfaction with family functioning) factors as more strongly related to depressive symptoms than the distal healthcare system factor (CCM services). Self-reported pain was the only ecological factor associated with anxiety symptoms. Conclusions Study findings provide a potential foundation for multi-factor screening of young adults with SB at risk for psychological symptoms.

The goals of the study included empirical examination of the utility of the Immediate and Post-Concussion Assessment and Cognitive Testing (ImPACT) test with adolescents who are deaf or hard-of-hearing and to investigate patterns of performance at baseline that may arise in the assessment of this population. Baseline assessment of student-athletes has been conducted on a widespread scale with focus on performance of typically developing student-athletes and some clinical groups, though to date no studies have examined adolescents who are deaf or hard-of-hearing.Retrospective and de-identified ImPACT baseline test used with deaf and hard-of-hearing high-school student-athletes (N = 143; 66% male, mean age = 16.11) was examined.Review indicated significant differences in some composite scores between the deaf and hard-of-hearing group and hearing normative comparisons. A possible marker of task misunderstanding was identified to occur more frequently within the deaf and hard-of-hearing sample (13% in deaf sample vs. .31% in hearing sample).Results may provide support for the consideration and use of additional measures to ensure comprehension of task demands when considering this tool for use with deaf and hard-of-hearing adolescents.

The present study examined the intellectual and adaptive functioning in a sample of children and young adults with Sturge-Weber Syndrome (SWS). A total of 80 research participants from a SWS study database underwent full neurological evaluation as part of their participation or concurrent medical care. Twenty-nine of the participants received neuropsychological evaluations. Analyses indicated no significant demographic or neurological differences between those who did and did not receive neuropsychological evaluations. Overall, the neuropsychological evaluation sample displayed significantly lower functioning relative to published normative data across domains of intellectual and adaptive functioning. Thirty-two percent of the sample displayed impaired performance (standard score ≤ 75) in intellectual functioning and 58% displayed impaired performance in adaptive functioning. Hemiparesis status independently predicted overall adaptive functioning while seizure frequency independently predicted overall intellectual functioning. Younger participants displayed significantly higher (more intact) ratings in adaptive functioning compared to older participants, specifically in overall adaptive functioning, motor skills, and community living skills. A composite measure of neurological status (SWS-NRS) incorporating seizure and hemiparesis status effectively distinguished between individuals with impaired or nonimpaired adaptive and intellectual functioning and showed promise as a screening method for identifying individuals with more involved intellectual and/or adaptive needs.

Myelomeningocele is often accompanied by hydrocephalus (MMH), making it a potentially unstable neurological condition requiring shunt placement and possible revisions. Serial neuropsychological assessment is an important tool in monitoring children with MMH, as cognitive changes can indicate shunt malfunction and hydrocephalus. We present the case of a girl with MMH who had five neuropsychological assessments (ages 5, 7, 11, 12, and 14). Despite a lack of overt neurological symptoms or report of behavioral decline, testing at age 11 revealed decline in multiple neurobehavioral domains, and imaging at that time showed increased hydrocephalus, requiring shunt revision. Subsequent neuropsychological assessment conducted after a 2-year period of medical stability showed improvement and/or a return to baseline levels in some skill areas (i.e., working memory, verbal memory, visuomotor integration, and sustained attention), yet more lasting impairments in others (i.e., Verbal IQ, processing speed, organization, and response inhibition). These lasting cognitive deficits potentially impact independent completion of complex medical self-care tasks. This pattern of recovery highlights vulnerability of brain systems supporting executive functions in children with hydrocephalus and shunt failure.

Parent and self-report ratings were obtained for 28 adolescents with myelomeningocele and congenital hydrocephalus using the Behavior Assessment System for Children (BASC; Reynolds & Kamphaus, 1998a, 1998b) and the Behavior Rating Inventory of Executive Function (BRIEF; Gioia, Isquith, Guy, & Kenworthy, 2000; Guy, Gioia, & Isquith, 1998). Parents rated their children as having significantly more problems, compared to published norms, on the BRIEF but not the BASC. Adolescents rated themselves as having significantly more problems, compared to parent ratings, on scales comprising the BRIEF Behavioral Regulation Index; but not on common scales of the BASC. Parents also reported more problems on the BRIEF Global Executive Composite than on all three primary BASC indices, and more problems on the BRIEF Metacognition Index than on the BRIEF Behavioral Regulation Index. We conclude that the BRIEF captures salient executive dysfunction among individuals with myelomeningocele and hydrocephalus, and may be useful in identifying needs for intervention that might not be identified by broad-band behavior rating scales alone.

OBJECTIVE To provide a description of an emerging neuroimaging methodology, near-infrared spectroscopy (nIRS), and a potential educational application of the unique aspects of this technology. SUMMARY nIRS is documented for its potential as a personal, portable, brain imaging system that may prove useful for cerebral monitoring in applied settings such as home, school, and work. The basis of nIRS brain imaging is reviewed, with summary descriptions of optical and neurovascular issues as well as a brief comparison to other brain imaging methodologies. Recent developments in nIRS technology are discussed, including ongoing validation efforts and potential applications for neuropsychologists. We describe one potential application of nIRS (i.e., educational neuroimaging) as an illustration of the use of nIRS technology and the potential expansion of the neuropsychologist's role in the educational setting. CONCLUSION nIRS holds the potential of opening new clinical questions and opportunities for neuropsychologists, and may provide a low-cost means of repeatable, neurovascular monitoring in nonmedical settings.

BACKGROUND AND OBJECTIVE: Behavioral disorders are highly comorbid with childhood learning disabilities (LDs), and accurate identification of LDs is vital for guiding appropriate interventions. However, it is difficult to conduct comprehensive assessment of academic skills within the context of primary care visits, lending utility to screening of academic skills via informant reports. The current study evaluated the clinical utility of a parent-reported screening measure in identifying children with learning difficulties. METHODS: Participants included 440 children (66.7% male), ages 5.25 to 17.83 years (mean = 10.32 years, SD = 3.06 years), referred for neuropsychological assessment. Academic difficulties were screened by parent report using the Colorado Learning Difficulties Questionnaire (CLDQ). Reading and math skills were assessed via individually administered academic achievement measures. Sensitivity, specificity, classification accuracy, and conditional probabilities were calculated to evaluate the efficacy of the CLDQ in predicting academic impairment. RESULTS: Correlations between the CLDQ reading scale and reading achievement measures ranged from −0.35 to −0.65 and from −0.24 to −0.46 between the CLDQ math scale and math achievement measures (all P < .01). Sensitivity was good for both reading and math scales, whereas specificity was low. Taking into account the high base rate of reading and math LDs within our sample, the conditional probability of true negatives (96.2% reading, 85.1% math) was higher than for true positives (40.5% reading, 37.9% math). CONCLUSIONS: Overall, the CLDQ may more accurately predict children without LDs than children with LDs. As such, the absence of parent-reported difficulties may be adequate to rule out an overt LD, whereas elevated scores likely indicate the need for more comprehensive assessment. * Abbreviations: ADHD — : attention-deficit/hyperactivity disorder CLDQ — : Colorado Learning Difficulties Questionnaire LD — : learning disability WIAT-III — : Wechsler Individual Achievement Test, Third Edition WJ-III — : Woodcock-Johnson Tests of Achievement, Third Edition

Kennedy Krieger Institute, Baltimore, Maryland and Johns Hopkins University Purpose/Objective: Successful implementation of functional self-care skills depends upon adequate executive functioning; however, many scales assessing adaptive skills do not address the inherent executive burden of these tasks. This omission is especially relevant for individuals with spina bifida, for whom medical self-care tasks impose a significant burden requiring initiation and prospective memory. The Kennedy Krieger Independence Scales-Spina Bifida Version (KKIS-SB) is a caregiver-reported measure designed to address this gap; it assesses skills for managing both typical and spina bifida-related daily self-care demands, with a focus on the timely and independent initiation of adaptive skills. Research Method/Design: Parents of 100 youth and young adults with spina bifida completed the KKIS-SB. Exploratory factor analysis and Pearson's correlations were used to assess the factor structure, reliability, and construct validity of the KKIS-SB. Results: The scale demonstrates excellent internal consistency (Cronbach's alpha .891). Exploratory factor analysis yielded four factors, explaining 65.1% of the total variance. Two primary subscales were created, initiation of routines and prospective memory, which provide meaningful clinical information regarding management of a variety of typical (e.g., get up on time, complete daily hygiene routines on time) and spina bifida-specific self-care tasks (e.g., begin self-catheterization on time, perform self-examination for pressure sores). Conclusions/ Implications: Based upon internal consistency estimates and correlations with measures of similar constructs, initial data suggest good preliminary reliability and validity of the KKIS-SB.

Objective To explore psychological symptoms in emerging adults with spina bifida (SB) and their association with self-management and satisfaction with family functioning. Methods Longitudinal data were collected at 2 time points, 15 months apart, in 48 individuals with SB. Reliable change indices and paired samples t-tests assessed change in anxiety and depressive symptoms. Hierarchical regression models explored the contributions of SB severity, family satisfaction, and self-management in explaining change in psychological symptoms. Results No significant group level differences in psychological symptoms were found across time in participants (Mean age 22 years), but significant individual-level change in anxiety symptoms (n ¼ 13) and depressive symptoms (n ¼ 9) was observed. Improved satisfaction with family functioning was associated with decreased anxiety symptoms (b ¼� 0.30, p ¼ .02), and increased SB self-management was related to reduced depressive symptoms (b ¼� 0.63, p ¼ .01). Conclusions Changes in self-management and satisfaction with family functioning may influence the course of psychological symptoms.

"Sluggish cognitive tempo" (SCT) is a construct hypothesized to describe a constellation of behaviors that includes daydreaming, lethargy, drowsiness, difficulty sustaining attention, and underactivity. Although the construct has been inconsistently defined, measures of SCT have shown associations with symptoms of attention-deficit/hyperactivity disorder (ADHD), particularly inattention. Thus, better characterization of SCT symptoms may help to better predict specific areas of functional difficulty in children with ADHD. The present study examined psychometric characteristics of a recently developed 14-item scale of SCT (Penny et al., Psychological Assessment 21:380-389, 2009), completed by teachers on children referred for outpatient neuropsychological assessment. Exploratory factor analysis identified three factors in the clinical sample: Sleepy/Sluggish, Slow/Daydreamy, and Low Initiation/Persistence. Additionally, SCT symptoms, especially those loading on the Sleepy/Sluggish and Slow/Daydreamy factors, correlated more strongly with inattentive than with hyperactive/impulsive symptoms, while Low Initiation/Persistence symptoms added significant unique variance (over and above symptoms of inattention) to the predictions of impairment in academic progress.

This special issue of The Clinical Neuropsychologist focuses on advances in the emerging subspecialty of pediatric neuropsychology. The national and international contributions in this issue cover a range of key clinical, research, training, and professional issues specific to pediatric neuropsychology. The genesis for this project developed out of a series of talks at the Philadelphia Pediatric Neuropsychology Symposium in 2010, hosted by the Stein Family Fellow, the Department of Psychology of the College of Arts and Sciences at Drexel University, and the Philadelphia Neuropsychology Society. Articles that explore clinical practice issue focus on the assessment of special medical populations with congenital and/or acquired central nervous system insults. Research articles investigate the core features of developmental conditions, the use of technology in neuropsychological research studies, and large sample size genomic, neuropsychological, and imaging studies of under-represented populations. The final series of articles examine new considerations in training, advocacy, and subspecialty board certification that have emerged in pediatric neuropsychology. This introductory article provides an overview of the articles in this special issue and concluding thoughts about the future of pediatric neuropsychology.

Sturge-Weber syndrome is a rare congenital disorder. Seizures, stroke-like episodes, glaucoma, headache, and developmental delay are frequently associated features. An Internet-based questionnaire was designed to assess the frequency of use, effectiveness, and safety of aspirin treatment in Sturge-Weber syndrome. Thirty-four of 98 subjects who completed the survey reported having used aspirin. The mean number of reported stroke-like episodes was reduced from 1.1 to 0.3 per month in the year after starting aspirin (n = 26, p = .014). The median number of seizures was significantly reduced from 3 to 1 episodes per month (n = 21, p = .002). Thirty-nine percent of subjects reported a history of complications (predominantly increased bruising or gum/nose bleeding) while on aspirin; however, none reported discontinuing aspirin because of side effects. Our study showed a significant relative reduction in both self-reported seizure frequency and stroke-like episodes after starting aspirin. It also suggests that low-dose aspirin can be safely used in these patients.

To advance understanding of the interrelationships of sex, level of lesion (LOL), self-management, community integration (employment, independent living), and quality of life (QOL) in young adults with myelomeningocele.A multicenter convenience sample of 50 individuals with myelomeningocele, 18 to 25 years of age (mean age 21 y 5 mo, SD 2 y), participated in a structured clinical interview on self-management (Adolescent Self-Management and Independence Scale II [AMIS II]) and completed a self-report questionnaire comprising standardized measures. QOL was assessed using the World Health Organization Quality of Life (WHOQOL)-BREF instrument. A chart review yielded clinical data.Most participants were Caucasian (78%), female (56%: 28 females, 22 males), unemployed (58%), and in supervised living environments (74%). Eighty per cent had a history of hydrocephalus requiring shunt placement. A lumbar LOL was most frequently reported (64%), followed by a sacral LOL (22%), and thoracic LOL (7%). Males were more likely to report employment (p=0.008), but females had greater success in transitioning into independent living settings (p=0.015). LOL was a significant predictor of specific dimensions of self-management, employment, and QOL (p0.05). Mean scores on the AMIS II reflected deficits in condition management activities and tasks of everyday life. Limited QOL was also observed.The overall low rates of employment and independent living suggest that individuals with myelomeningocele are experiencing great difficulty in achieving these milestones of emerging adulthood, regardless of sex. Limited success in developing self-management skills and restricted QOL also highlight vulnerability in this population.

The Life Course Model for patients, families, caregivers, teachers, and clinicians was developed with support by the National Spina Bifida Program, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, for individuals with spina bifida. The Life Course Model facilitates a developmental approach to assessment and intervention along life's trajectory. This Life Course Model provides information about key developmental milestones for particular age groups, validated assessments that can be performed by clinicians or teachers to determine if milestones have been reached, useful suggestions for intervening in creative ways at each step, and evidence-based references. In this article, the authors introduce the viewpoints of several key clinicians who are involved in the care of individuals with spina bifida and how the Life Course Model can assist them, their patients, and their families in the process of assessment, intervention, collaboration with other clinicians, and follow-up. A case study is used to demonstrate the experience of comprehensive and collaborative management in transitioning a child and his family from infancy to adulthood.

The transition of youth with spina bifida into adulthood is an exciting opportunity to branch out, explore and participate in community, and reach higher levels of independence. The Life Course Model Web site is a resource designed to help in this process. This article describes how this tool can help individuals with spina bifida, parents, teachers, and caregivers evaluate this process and provide assistance where necessary.

Sturge-Weber Syndrome (SWS) is a rare neurocutaneous disorder involving facial capillary malformation (port-wine birthmark) and vascular malformation of the brain that is frequently associated with epilepsy, stroke-like episodes, cognitive deficits, motor impairment, and/or visual field cut. The four cases presented here (ages 8-9, two females) illustrate the broad range of physiologic involvement and associated neuropsychological functioning in SWS, and argue against the idea of a "typical" SWS neuropsychological presentation. Rather, we highlight a preliminary collection of disease status/severity factors thought to impact neuropsychological presentation in SWS, including degree of cortical involvement (unilateral versus bilateral; posterior only versus posterior/anterior), age at time of seizure onset, extent of seizure control, history of stroke-like episodes, and magnitude of neurologic decline/deficit. We discuss the need for broad-based assessment in this medical population, as various impairment combinations (e.g., perceptual, language, executive) create unique presentations as well as the need for individualized intervention.

Assessment of attentional processes via computerized assessment is frequently used to quantify intra-individual cognitive improvement or decline in response to treatment. However, assessment of intra-individual change is highly dependent on sufficient test reliability. We examined the test–retest reliability of selected variables from one popular computerized continuous performance test (CPT)—i.e., the Conners’ CPT – Second Edition (CPT-II). Participants were 39 healthy children (20 girls) ages 6–18 without intellectual impairment (mean PPVT-III SS = 102.6), LD, or psychiatric disorders (DICA-IV). Test–retest reliability over the 3–8 month interval (mean = 6 months) was acceptable (Intraclass Correlations [ICC] = .82 to .92) on comparison measures (Beery Test of Visual Perception, WISC-IV Block Design, PPVT-III). In contrast, test–retest reliability was only modest for CPT-II raw scores (ICCs ranging from .62 to .82) and T-scores (ICCs ranging from .33 to .65) for variables of interest (Omissions, Commissions, Variability, Hit Reaction Time, and Attentiveness). Using test–retest reliability information published in the CPT-II manual, 90% confidence intervals based on reliable change index (RCI) methodology were constructed to examine the significance of test–retest difference/change scores. Of the participants in this sample of typically developing youth, 30% generated intra-individual changes in T-scores on the Omissions and Attentiveness variables that exceeded the 90% confidence intervals and qualified as “statistically rare” changes in score. These results suggest a considerable degree of normal variability in CPT-II test scores over extended test–retest intervals, and suggest a need for caution when interpreting test score changes in neurologically unstable clinical populations.

This study sought to identify neurologic correlates of adaptive functioning in individuals with Sturge-Weber syndrome. A total of 18 children, adolescents, and young adults with Sturge-Weber syndrome with brain involvement were recruited from our Sturge-Weber center. All underwent neurologic examination (including review of clinical brain magnetic resonance imaging) and neuropsychological assessment. Neuropsychological assessment included measures of intellectual ability and standardized parent report of adaptive functioning. Overall, Full Scale IQ and ratings of global adaptive functioning were both lower than the population-based norms (P < .05). Negative correlations were identified between adaptive functioning ratings, clinician ratings of cortical abnormality, and ratings of neurologic status. Hemiparesis (minimal versus prominent) was the only individual component of the rating scales that differentiated between individuals with nonimpaired and impaired adaptive functioning scores. Information obtained during neurological examination of children and adolescents with Sturge-Weber syndrome particularly hemiparetic status is useful for identifying children who may need additional intervention.

Previous research has suggested that adolescents with myelomeningocele and shunted hydrocephalus (MMH) have difficulties with aspects of executive functioning and, in turn, with functional independence. There is little research, however, examining patterns of executive functioning across adolescence in this population. The goal of this cross-sectional study was to examine parent ratings of executive function in children with MMH and in typically developing peers across late childhood and adolescence. Parents of 36 individuals with MMH and 35 typically developing peers, ages 10 to 18 years, completed the Behavior Rating Inventory of Executive Function (BRIEF). The BRIEF is organized into eight scales and two primary indices—Metacognition (MCI) and Behavioral Regulation (BRI). As a whole, the children with MMH had significantly higher BRIEF T-scores, as well as a higher prevalence of clinically significant T-scores across subscales, particularly those representing cognitive control. Effects of group, age, and age-by-group interactions on the mean raw scores of the MCI and BRI were examined using regression analyses. There were significant group effects (p < .05) for both the BRI and MCI, with the controls having significantly lower mean ratings than the MMH group. There was also a significant contribution of age-by-group interaction on the BRI (p < .05). Although mean raw scores on the BRI for the MMH group remained stable across ages, mean raw scores in the control group decreased as age increased. Thus, healthy children have age-related improvements in executive control behaviors across adolescence, particularly behavioral control, while children with MMH demonstrate no age-related improvements in parent reported executive behaviors across adolescence. Therefore, children with MMH may continue to require targeted interventions and modifications to address executive dysfunction into young adulthood in order to promote functional independence.

Address: 1Neurology & Developmental Medicine, Keelty Center for Spina Bifida, Kennedy Krieger Institute, North Broadway, Baltimore, Maryland, USA, 2Behavioral Psychology, Keelty Center for Spina Bifida, Kennedy Krieger Institute, North Broadway, Baltimore, Maryland, USA, 3Neuropsychology, Kennedy Krieger Institute, Keelty Center for Spina Bifida, Kennedy Krieger Institute, North Broadway, Baltimore, Maryland, USA, 4Urology, Johns Hopkins Hospital, Baltimore, Maryland, USA and 5School of Nursing, University of Maryland, Baltimore, Maryland, USA